Calandrelli Rosalinda, Mallio Carlo Augusto, Bernetti Caterina, Pilato Fabio
Advanced Radiology Center (ARC), Department of Oncological Radiotherapy, and Hematology, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, 00168 Rome, Italy.
Research Unit of Diagnostic Imaging and Interventional Radiology, Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, Via Alvaro del Portillo, 21, 00128 Rome, Italy.
Diagnostics (Basel). 2025 Jan 31;15(3):334. doi: 10.3390/diagnostics15030334.
Multinodular and vacuolating neuronal tumors (MNVTs) are a type of recently identified benign neuroepithelial tumor with debated malformative or neoplastic origins. This review summarizes their neuroanatomical localization, imaging, histopathology, immunohistochemistry, and diagnostic challenges. : A systematic review of PUBMED/MEDLINE was performed in December 2024. Of 118 screened articles, 39 were eligible, covering 299 patients. MNVTs are often asymptomatic "leave me alone" lesions, discovered incidentally, though nonspecific symptoms (59.9%) and seizures (19.7%) are reported. Immunohistochemistry reveals variable profiles, reflecting complex cellular differentiation. The characteristic "bubble-like" MRI pattern along the subcortical ribbon and superficial white matter is a reliable diagnostic feature. Rare cortical involvement and atypical band-like lesions occur. MRI signal intensity varies. Over a mean follow-up of 36 months, lesions were stable or non-recurrent, with only one case of progression. MVNT imaging mimics other glioneuronal lesions, but reliable diagnostic MRI features include a "bubble-clustered" appearance, lack of cortical involvement, absence of enhancement, and temporal lobe predominance. Hemodynamic and metabolic properties support the diagnosis. Most lesions remain stable, requiring no treatment. Surgical resection is reserved for cases with uncontrolled seizures or atypical locations where the diagnosis is unclear.
多结节空泡状神经元肿瘤（MNVTs）是一种最近才被发现的良性神经上皮肿瘤，其起源是发育异常还是肿瘤性存在争议。本综述总结了它们的神经解剖定位、影像学、组织病理学、免疫组化及诊断挑战。2024年12月对PUBMED/MEDLINE进行了系统综述。在筛选的118篇文章中，39篇符合条件，涵盖299例患者。MNVTs通常是无症状的“无需处理”病变，多为偶然发现，不过也有非特异性症状（59.9%）和癫痫发作（19.7%）的报道。免疫组化显示出不同的特征，反映了复杂的细胞分化。沿皮质下带状区和浅表白质的特征性“气泡样”MRI表现是可靠的诊断特征。罕见的皮质受累及非典型带状病变也会出现。MRI信号强度各不相同。平均随访36个月期间，病变稳定或无复发，仅1例进展。MVNT的影像学表现与其他神经胶质神经元病变相似，但可靠的诊断性MRI特征包括“气泡聚集”外观、无皮质受累、无强化及颞叶优势。血流动力学和代谢特性支持诊断。大多数病变保持稳定，无需治疗。手术切除适用于癫痫无法控制或诊断不明确的非典型部位的病例。
